A 72-year-old woman was referred to our hospital for palliative care. Fifteen years earlier, she had undergone total hysterectomy and radiotherapy for cervical cancer. One year before her referral, she visited a hospital due to a gait disturbance and was diagnosed with lymphangiosarcoma. The level of coagulation factor VIII plasma activity was >201% (normal range: 62-145%) and the immunohistochemical results were positive for factor VIII-related antigen in a tumor specimen. To the best of our knowledge, this is the first report of high coagulation factor VIII plasma activity in a patient with lymphangiosarcoma.